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Purpose: To assess and compare intrareader and interreader reproducibility of magnetic resonance imaging (MRI) diagnosis of female genital anomalies (FGAs) using the American Society for Reproductive Medicine-Mullerian anomalies classification 2021 (ASRM-MAC 2021) and European Society of Human Reproduction and Embryology-European Society for Gynecological Endoscopy (ESHRE-ESGE) 2016 classification. Methods: In this retrospective study, we searched our electronic MRI database from April 2021 to September 2023, selecting MRI studies with FGAs. Seventy-six consecutive studies were included and reviewed by 4 independent radiologists using both classifications. Studies were re-evaluated after 1 month. Reproducibility was assessed using kappa (κ) scores with 95% confidence intervals (CI). Results: Intrareader agreement for MRI diagnosis of FGAs was substantial to excellent, with κ scores ranging from 0.684 (95% CI, 0.534-0.834) to 0.985 (95% CI, 0.963-1.01) using the ASRM-MAC 2021 and from 0.743 (95% CI, 0.621-0.865) to 0.846 (95% CI, 0.719-0.973) using the ESHRE-ESGE 2016 classification. Pairwise interreader agreement was higher with the ASRM-MAC 2021, ranging from moderate (κ = 0.491; 95% CI, 0.341-0.642) to substantial (κ = 0.709; 95% CI, 0.597-0.821), compared to the ESHRE-ESGE 2016 classification, with weak (κ = 0.080; 95% CI, 0.068-0.228) to moderate (κ = 0.511; 95% CI, 0.344-0.678) agreement. Overall interreader agreement was moderate for both classifications (κ = 0.599; 95% CI, 0.562-0.638 for ASRM-MAC 2021 and κ = 0.429; 95% CI, 0.396-0.463 for ESHRE-ESGE 2016 classification), but with significant differences (non-overlapping CIs). Conclusion: The intrareader reproducibility was high for both classifications, whereas the interreader reproducibility was higher using the ASRM-MAC 2021, highlighting the impact of classification criteria on the reproducibility of MRI diagnosis of FGAs. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study is to analyze the precise anatomical characteristics of a large group of patients with Herlyn-Werner-Wunderlich syndrome together with the long-term follow-up and the reproductive performance.Study Design: Retrospective analysis of prospectively collected data. In two tertiary medical centers, from 2008 to 2021, 51 patients with HWWS underwent surgery via the same technique. Presenting symptoms, preoperative investigations, operative management, and long-term follow-up with obstetric outcome were assessed.Results: The surgical procedure was successful in all cases with no major complications recorded. Postoperative course was uneventful. The median follow-up was 6,5 years, with a range from 6 months to 13 years. After surgery, among 14 patients who sought pregnancies, 12 (85,71%) were successful. Of these 11 patients had a total of 22 pregnancies, resulting in spontaneous miscarriage in 27% (6/22), premature birth (<37 weeks) in 36% (8/22) and full-term birth in 36% (8/22). Overall the pregnancies demonstrated had a good course.Conclusions: Early diagnosis is essential in establishing prompt and correct surgical treatment. [ABSTRACT FROM AUTHOR]

Introduction: The first-line approach for the management of distal vaginal atresia involves a pull-through vaginoplasty. If the proximal vagina is 3 cm or more from the introitus, the risk of stenosis is high, and an interposition graft may be necessary. We describe a safe, low-cost, and accessible approach for distal vaginal atresia ≥ 3 cm that we call the "modified balloon vaginoplasty" and validate the technical feasibility and anatomical outcomes.Methods: Ten patients who underwent modified balloon vaginoplasty were retrospectively evaluated. Age, symptoms at presentation, length of atresia, operation time, and postoperative complications were analyzed.Results: All the cases were successfully performed without any intraoperative morbidity. The postoperative complications included one case of stenosis ring in the distal vagina because not right used vagina model. All the girls had regular menstruation and were satisfied with the surgical outcome.Conclusion: Modified balloon vaginoplasty allows further distention of the distal vagina or thinning of the septum, which may decrease the risk of stenosis, is a beneficial choice for patients with distal vaginal atresia ≥ 3 cm. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

Study Objective To assess the clinical course of obstructive Müllerian anomalies found in girls after menarche. Design A retrospective case series of adolescents who, between 2009 and 2016, were treated for vaginal or uterine obstructive malformations diagnosed after menarche. Setting Division of Gynecology, Poznań University of Medical Sciences, Poznań, Poland. Participants and Interventions Twenty-two patients who, at the age range between 11.4 and 18.2 (median, 13.1) years, between 2 and 74 (median 7.5) months after menarche, underwent surgical repair of obstructive genital anomaly. Main Outcome Measures Müllerian defect type, presentation, radiologic findings, pre- and postoperative course. Results Eighteen patients (18 of 22; 81.8%) were diagnosed with obstructed hemivagina ipsilateral renal anomaly syndrome. One patient (1 of 22; 4.5%) was diagnosed with uterus didelphys and unilateral cervical atresia. Three patients (3 of 22; 13.6%) had unicornuate uterus with a cavitated, noncommunicating rudimentary horn. The right side was affected in 13 patients (13 of 22; 59.1%), and the left side in 9 patients (9 of 22; 40.9%; P > .05). All but 1 patient had renal agenesis on the side of obstruction. Before repair of the obstructive genital anomaly, 4 patients underwent unnecessary surgeries for misdiagnosed ovarian cysts. Serious complications (pelvic inflammatory disease, vesicovaginal fistula) occurred in 2 patients with microperforated pyocolpos. Pelvic endometriosis was found in 4 of our patients. Conclusion Our case series suggests that obstructed hemivagina ipsilateral renal anomaly syndrome is the most common obstructive Müllerian anomaly diagnosed in adolescents after menarche. The differential diagnosis for unilateral kidney agenesis accompanied by dysmenorrhea in adolescent girls should include obstructive genital tract anomaly. Accurate diagnosis of an obstructive genital anomaly early after menarche might help prevent unnecessary surgeries and infection-related complications. Meanwhile, prompt surgical correction of an obstructive genital tract anomaly results in relief of symptoms and might reduce the risk of endometriosis. [ABSTRACT FROM AUTHOR]

What is the recommended diagnostic work-up of female genital anomalies according to the European Society of Human Reproduction and Embryology (ESHRE)/European Society for Gynaecological Endoscopy (ESGE) system? The ESHRE/ESGE consensus for the diagnosis of female genital anomalies is presented. Accurate diagnosis of congenital anomalies still remains a clinical challenge due to the drawbacks of the previous classification systems and the non-systematic use of diagnostic methods with varying accuracy, with some of them quite inaccurate. Currently, a wide range of non-invasive diagnostic procedures are available, enriching the opportunity to accurately detect the anatomical status of the female genital tract, as well as a new objective and comprehensive classification system with well-described classes and sub-classes. The ESHRE/ESGE Congenital Uterine Anomalies (CONUTA) Working Group established an initiative with the goal of developing a consensus for the diagnosis of female genital anomalies. The CONUTA working group and imaging experts in the field have been appointed to run the project. The consensus is developed based on (1) evaluation of the currently available diagnostic methods and, more specifically, of their characteristics with the use of the experts panel consensus method and of their diagnostic accuracy performing a systematic review of evidence and (2) consensus for (a) the definition of where and how to measure uterine wall thickness and (b) the recommendations for the diagnostic work-up of female genital anomalies, based on the results of the previous evaluation procedure, with the use of the experts panel consensus method. Uterine wall thickness is defined as the distance between interostial line and external uterine profile at the midcoronal plane of the uterus; alternatively, if a coronal plane is not available, the mean anterior and posterior uterine wall thickness at the longitudinal plane could be used. Gynaecological examination and two-dimensional ultrasound (2D US) are recommended for the evaluation of asymptomatic women. Three-dimensional ultrasound (3D US) is recommended for the diagnosis of female genital anomalies in 'symptomatic' patients belonging to high-risk groups for the presence of a female genital anomaly and in any asymptomatic woman suspected to have an anomaly from routine avaluation. Magnetic resonance imaging (MRI) and endoscopic evaluation are recommended for the sub-group of patients with suspected complex anomalies or in diagnostic dilemmas. Adolescents with symptoms suggestive for the presence of a female genital anomaly should be thoroughly evaluated with 2D US, 3D US, MRI and endoscopy. The various diagnostic methods should be used in a proper way and evaluated by experts to avoid mis-, over- and underdiagnosis. The role of a combined ultrasound examination and outpatient hysteroscopy should be prospectively evaluated. It is a challenge for further research, based on diagnosis, to objectively evaluate the clinical consequences related to various degrees of uterine deformity. [ABSTRACT FROM AUTHOR]

Study Question: What is the recommended diagnostic work-up of female genital anomalies according to the European Society of Human Reproduction and Embryology (ESHRE)/European Society for Gynaecological Endoscopy (ESGE) system?Summary Answer: The ESHRE/ESGE consensus for the diagnosis of female genital anomalies is presented.What Is Known Already: Accurate diagnosis of congenital anomalies still remains a clinical challenge because of the drawbacks of the previous classification systems and the non-systematic use of diagnostic methods with varying accuracy, some of them quite inaccurate. Currently, a wide range of non-invasive diagnostic procedures are available enriching the opportunity to accurately detect the anatomical status of the female genital tract, as well as a new objective and comprehensive classification system with well-described classes and sub-classes.Study Design, Size, Duration: The ESHRE/ESGE CONgenital UTerine Anomalies (CONUTA) Working Group established an initiative with the goal of developing a consensus for the diagnosis of female genital anomalies. The CONUTA working group and imaging experts in the field have been appointed to run the project.Participants/materials, Setting, Methods: The consensus is developed based on: (i) evaluation of the currently available diagnostic methods and, more specifically, of their characteristics with the use of the experts panel consensus method and of their diagnostic accuracy by performing a systematic review of evidence and (ii) consensus for the definition of where and how to measure uterine wall thickness and the recommendations for the diagnostic work-up of female genital anomalies, based on the results of the previous evaluation procedure, with the use of the experts panel consensus method.Main Results and the Role Of Chance: Uterine wall thickness is defined as the distance between the interostial line and external uterine profile at the midcoronal plane of the uterus; alternatively, if a coronal plane is not available, the mean anterior and posterior uterine wall thickness at the longitudinal plane could be used. Gynecological examination and two-dimensional ultrasound (2D US) are recommended for the evaluation of asymptomatic women. Three-dimensional (3D) US is recommended for the diagnosis of female genital anomalies in 'symptomatic' patients belonging to high risk groups for the presence of a female genital anomaly and in any asymptomatic woman suspected to have an anomaly from routine evaluation. Magnetic resonance imaging (MRI) and endoscopic evaluation are recommended for the subgroup of patients with suspected complex anomalies or in diagnostic dilemmas. Adolescents with symptoms suggestive for the presence of a female genital anomaly should be thoroughly evaluated with 2D US, 3D US, MRI and endoscopically.Limitations, Reasons For Caution: The various diagnostic methods should always be used in the proper way and evaluated by experts to avoid mis-, over- and underdiagnosis.Wider Implications Of the Findings: The role of a combined US examination and outpatient hysteroscopy should be prospectively evaluated. It is a challenge for further research, based on diagnosis, to objectively evaluate the clinical consequences related to various degrees of uterine deformity.Study Funding/competing Interests: None. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: To present the first repeat procedure and a simple way for management of balloon rupture during balloon vaginoplasty (BV) procedures. Study design: Repeat BV (reBV) procedure was done for one case, and a ruptured catheter''s end to new catheter''s end (ENE) procedure was done for two cases. The anatomic and functional outcomes of reBV and ENE are presented. Results: Balloon rupture and deflation were encountered in a total of 3/45 cases who had undergone BV procedures. Replacement time was 22min for reBV, and 9 and 10min for ENE. General anesthesia was needed in the reBV case. Post-operative course and final outcomes were generally good but objectively less favorable in the ENE than in the reBV case (neovaginal depths were 8.5 and 9.4cm versus 11cm, respectively). Penetration and satisfaction scores were increased up 85 points for both couples. Conclusions: Balloon ruptures were reported in 3/45 cases undergoing balloon vaginoplasty. End to new catheter end replacement was a very fast and simple rescue procedure but its preliminary anatomical outcomes were less favorable than the outcomes obtained with the original or repeat procedure. [Copyright &y& Elsevier]

Current proposals for classifying female genital anomalies seem to be associated with limitations in effective categorization, creating the need for a new classification system that is as simple as possible, clear and accurate in its definitions, comprehensive, and correlated with patients'' clinical presentation, prognosis, and treatment on an evidence-based foundation. Although creating a new classification system is not an easy task, it is feasible when taking into account the experience gained from applying the existing classification systems, mainly that of the American Fertility Society. [Copyright &y& Elsevier]

Inverted balloon vaginoplasty technique was performed for a case with segmental vaginal aplasia and upper hematocolpos within 50 min operative time without any complications. The neovagina was naturally covered, 11 cm in depth and communicated with dimple at hymen''s site. Regular painless menstruations were reported. [Copyright &y& Elsevier]

We evaluated the feasibility and operative and postoperative outcomes of office balloon vaginoplasty. Office balloon vaginoplasty was performed successfully for a case with vaginal aplasia. She was currently married and presenting with minimal penetration and dyspareunia. We successfully performed the procedure through retro-pubic space under local anesthesia within 25 min total operative time and it was well tolerated by the patient. No operative complications were reported. The resulting neovagina was cosmetically attractive and 10 cm in depth. Sexual intercourse was started on the day of catheter removal. Penetration and satisfaction scores increased up to 90 points for both partners. [ABSTRACT FROM AUTHOR]